title: Ectopic adrenal rests in congenital adrenal hyperplasia as a cause of androgen excess after adrenalectomy detected by pelvic venous sampling.
abstract: BACKGROUND
  Patients with classic congenital adrenal hyperplasia (CAH) due to CYP21 deficiency are treated with supraphysiological doses of glucocorticoids to suppress elevated androgen production. This implies also side effects of high-dose glucocorticoids, possibly leading to iatrogenic Cushing's syndrome. Bilateral adrenalectomy has been suggested as the ultimate therapy in severe cases, when insufficient androgen suppression was obtained despite high glucocorticoid doses. Usually, ACTH levels rise after bilateral adrenalectomy, and this could imply an increased risk for the development of ectopic adrenal rests. In female CAH patients ovarian adrenal rests are not commonly detected by conventional radiological techniques.
  
  
  METHODS
  We report the case of an adult female CAH patient who underwent bilateral adrenalectomy in early puberty because of poorly controlled CAH.
  
  
  CONCLUSIONS
  Several years after surgery, she developed secondary amenorrhea and hair loss as a result of androgen overproduction in ovarian adrenal rests that appeared to be detectable only by pelvic venous sampling. After unilateral oophorectomy androgen levels normalized.
authors.affiliation: Department of Pediatric Endocrinology, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands.
authors.forename: H L
authors.initials: HL
authors.lastname: Claahsen-van der Grinten
authors.forename: M M L
authors.initials: MM
authors.lastname: Stikkelbroeck
authors.forename: J
authors.initials: J
authors.lastname: Bulten
authors.forename: M
authors.initials: M
authors.lastname: den Heyer
mesh_terms: D000311:Adrenal Glands; D000312:Adrenal Hyperplasia, Congenital / Q000097:blood* / Q000601:surgery*; D000328:Adult; D000728:Androgens / Q000097:blood*; D002828:Choristoma / Q000601:surgery*; D005260:Female; D006801:Humans
chemical_list: D000728:Androgens